Detailed neuropathological findings in 222 cases of naturally occurring scrapie from Great Britain are described. The material consisted of formalin-fixed brain from eight breeds of sheep submitted between 1982 and 1991. Paraffin-embedded histological sections were made from several specified brain areas, including the medulla oblongata, cerebellum, pons, mesencephalon, diencephalon, septal area, basal ganglia and frontal cortex. Sections were examined by conventional and polarised light microscopy and the type and distribution of the lesions were recorded. Histologically, the lesions included vacuolation of neuronal perikarya and grey matter neuropil, neuronal degeneration (especially "dark' neurons) and loss, a reactive glial (predominantly astrocytic) response and amyloidosis. Vacuolar lesions were present in the cerebral cortex of 37 per cent of cases, centred around the superior frontal gyrus. Vacuolar lesions were detected in the neocortex for as long as sections have been taken from the superior frontal gyrus and are thus probably not a new feature of the disease. The distribution of vacuolation in the grey matter neuropil could be classified into seven patterns. Data from individual breeds of sheep showed that in some breeds there were significant differences in the age at which animals with different patterns of vacuolation died from scrapie.
Six cases of scrapie were confirmed in two separately maintained flocks of moufflon, in both of which the disease appeared to be endemic. The clinical signs and histopathology were indistinguishable from those observed in scrapie-affected domesticated sheep. The pathology included lesions in the cerebral cortex which, although commonly present in scrapie-affected sheep, have not previously been described in the natural disease.
The brains of the 20 goats affected with natural scrapie received at the Central Veterinary-Laboratory, Weybridge, since 1975 were examined microscopically. Lesions of a spongiform encephalopathy were found in the brainstem, cerebellum, diencephalon, corpus striatum, and also in the neopallium or cerebral cortex. The lesions in the neopallium have not previously been reported in natural scrapie in goats. Deposits of amyloid were present in the thalamus in three of the 20 goats.
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